Purtscher-like retinopathy associated with dermatomyositis

Purpose To describe a multimodal imaging diagnosis of retinopathy in dermatomyositis. Case presentation A 21-year-old white woman with a history of fatigue and a cutaneous rash complained of visual impairment in her left eye. A funduscopic examination showed multiple confluent cotton-wool spots in both eyes. Swept source-optical coherence tomography presented macular edema in both eyes; optical coherence tomography angiography revealed superficial and deep capillary occlusion in all areas affected by cotton-wool spots; and fluorescein angiography showed vascular walls enhancement, veins dilatation, and capillary leakage. After large doses of intravenously administered glucocorticoid therapy, followed by a cyclophosphamide regimen, best corrected visual acuity returned to 20/20 in both eyes. Conclusions This case report presents optical coherence tomography angiography clinical findings in a rare case of dermatomyositis-associated retinopathy, remarking the importance of a multi-imaging approach for a correct diagnosis and treatment of eye injuries, in order to avoid serious complications and permanent sequelae.

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“…Further studies classified this clinical appearance under the name of Purtscher-like retinopathy associated with DM [8].…”

Section: Introductionmentioning

confidence: 99%

Optical coherence tomography angiography in Purtscher-like retinopathy associated with dermatomyositis: a case report

et al. 2019

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“…The major cause of death in most cases was septic shock and multiorgan failure. Our patient received Rituximab early in course of disease (day 6) and started improvement within 48 h. We found only one adult DM patient who developed Purtscher's retinopathy (20). In JDM, there has been 1 case reported in France that developed TTP and Purtscher's retinopathy (11).…”

Section: Literature Review and Discussionmentioning

confidence: 70%

Juvenile Dermatomyositis (JDM) Complicated by Thrombotic Thrombocytopenic Purpura (TTP) and Purtscher's Retinopathy Responsive to Rituximab: Case Report and Literature Review

et al. 2020

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Juvenile dermatomyositis (JDM) is a multisystem vasculopathy that infrequently presents with acute complications (1). We report here the case of a 12-year-old girl with JDM who developed Thrombotic Thrombocytopenic purpura (TTP) and Purtscher's retinopathy. This is the second pediatric case of JDM with TTP and Purtscher's retinopathy in the literature. The diagnosis of JDM was based on her clinical presentation (fever, myalgia, proximal muscle weakness, characteristic skin rash and elevated muscle enzymes) (2). Despite improvement of rash, fever and weakness with corticosteroids and intravenous Immunoglobulins (IVIG), the patient developed retinopathy, thrombocytopenia, hemolytic anemia, renal failure, and pulmonary edema within 1 week of initial treatment. A clinical diagnosis of TTP and Purtscher's retinopathy was made and her ADAMTS13 activity was found to be low. Regardless of aggressive treatment with pulse steroid therapy, IVIG, plasmapheresis along with multiple infusions of Fresh Frozen plasma (FFP), her condition deteriorated. In view of her worsening condition, she received one dose of Rituximab and within 48 h, her hematological and retinal involvements improved. Rituximab was given at the same dose once weekly thereafter for 4 total doses. Her disease process was halted, and retinopathy improved significantly in 48 h and continued to gradually improve over 3 weeks of maintenance therapy with cyclosporine, methotrexate, and IVIG and then stabilized. This report documents the association of TTP and Purtscher's retinopathy with JDM, emphasizing that early recognition and prompt treatment with rituximab along with the current standard of care treatment i.e., Vincristine, corticosteroids and plasmapheresis could be of potential benefit in controlling disease activity.

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“…Retinal vasculitis is a rare complication of DM. The few published case reports describe retinal hemorrhages and cotton wool spots 5, 6, 7. This is a single published case report of severe vision loss from central retinal vein occlusion associated with DM 8 .…”

Section: Discussionmentioning

confidence: 79%

The pulseless patient: Profound vasculopathy as the presenting feature of fulminant dermatomyositis and response to therapy

et al. 2019

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Purtscher-like retinopathy associated with dermatomyositis

Cited by 20 publications

References 8 publications

Optical coherence tomography angiography in Purtscher-like retinopathy associated with dermatomyositis: a case report

Optical coherence tomography angiography in Purtscher-like retinopathy associated with dermatomyositis: a case report

Juvenile Dermatomyositis (JDM) Complicated by Thrombotic Thrombocytopenic Purpura (TTP) and Purtscher's Retinopathy Responsive to Rituximab: Case Report and Literature Review

The pulseless patient: Profound vasculopathy as the presenting feature of fulminant dermatomyositis and response to therapy

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